Acute cerebellitis in adults: a case report and review of the literature

• Main Authors: A. Van Samkar, M. N. F. Poulsen, H. P. Bienfait, R. B. Van Leeuwen
• Format: Article
• Language: English
• Published: BMC 2017-11-01
• Series: BMC Research Notes
• Subjects: Acute cerebellitis; Acute cerebellar ataxia; Hydrocephalus; Inflammation
• Online Access: http://link.springer.com/article/10.1186/s13104-017-2935-8

Abstract Background Acute cerebellitis is a rare disease with the majority of cases described in children. Little is known about the clinical characteristics and outcome in adults. Case presentation A 37-year-old Caucasian woman presented with headache, nausea, and photophobia, and was diagnosed as having a migraine attack. Two days later, she subsequently returned with aggravated headache, dysarthria and horizontal nystagmus. Magnetic resonance imaging (MRI) showed a swollen cerebellum and hydrocephalus and the patient was diagnosed with acute cerebellitis. Cerebrospinal fluid (CSF) examination showed an elevated leukocyte count and protein. Blood serology showed the presence of immunoglobulin M and immunoglobulin G for both Epstein–Barr virus and cytomegalovirus. The patient was treated with dexamethasone and discharged to a rehabilitation center, where she fully recovered. We searched the literature for adult cases of acute cerebellitis. Including our patient, we identified 35 patients with a median age of 36 years. The etiology was unknown in 34% of cases. The most common clinical presentation consisted of headache, nausea/vomiting and ataxia. Six patients presented with only headache and nausea and subsequently returned with cerebellar signs. In 9 cases, the cerebellitis was complicated by hydrocephalus. Half of the patients ended up with neurological sequelae, while follow-up MRI was abnormal in 71%. Conclusion Acute cerebellitis in adults is a rare disorder which mainly presents with headache, nausea/vomiting and ataxia. To diagnose cerebellitis, imaging of the brain (preferably MRI) is required and CSF examination may be necessary to narrow the differential diagnosis. The treatment depends on the widely diverse etiology, and treatment with steroids is recommended in the case of cerebellar oedema and hydrocephalus. Neurosurgical intervention may be necessary to prevent brain herniation.